CONGENITAL DIAPHRAGMATIC HERNIA ( C D H )

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CONGENITAL DIAPHRAGMATIC HERNIA ( C D H )

• Dr JACOB MATHEW
• DEPT. OF PAED. SURGERY
• Dr AHMED ABANAMY HOSPITAL

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NORMAL DIAPHRAGM
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C D H
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Development of diaphragm4th to 8th week
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Development of Lung
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Correlation of diaphragmatic defect and lung
development
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INCIDENCE

• 1 2000 3000
• MALE FEMALE
• 80 Left
• 20 Right
• B/L Rare
• Risk of recurrence in first degree relative is 2

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CAUSE

• We do not know
• Exposure to phenmetriazine, thalidomide, quinine,
  nitrofen and Vitamin A deficiency
• Genetic influences
• Associated with chromosomal deletion(XO)
  chromosomal duplication-Trisomy 21,18,13
• Most cases non-syndromatic, isolated

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PATHO-PHYSIOLOGY
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Patho-physio - contd
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Patho-physio - contd Pulmonary hypertension
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Patho-physio -contd

• Known stimulators of pulmonary hypertension
• Hypoxia
• Hypothermia
• Stress
• Acidosis

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Patho-physio contd persistence of (R) to (L)
shunting
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DIAGNOSISPrenatal diagnosis U/S
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DIAGNOSIS Prenatal diagnosis -MRI
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Post-natal diagnosis

• Respiratory symptoms at birth
• Respiratory symptoms within 24 hours
• Poor respiratory efforts, gasping
• Cyanosis, decreased peripheral perfusion
• Scaphoid abdomen
• Asymmetric funnel chest
• Bowel sounds in the chest
• 10 - 20 late presentation

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INVESTIGATIONFor diagnosis
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Investigation - contd
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Investigation associated anomalies

• Echocardiogram Cardiac defect
• Cranial U/S - Neural tube defects
• Abdomen U/S - Renal anomalies

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PROGNOSTIC FACTORS - Prenatal

• Lung to head ratio (LHR) gt1.4 Better
  prognosis, lt 1 very poor prognosis
• Liver position
• Position of stomach
• Prenatal diagnosis
• Polyhydramnios
• (R) sided defect

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Prognostic factors -Prenatal contd

• ASSOCIATED ANOMALIES Chromosomal anomalies and
  serious cardiac defects have a negative impact
  while defects like solitary kidney, mal-rotation
  have no bearing on the prognosis.

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PROGNOSTIC FACTORS - Postnatal

• PHYSIOLOGICAL PARAMETERS Blood gas analysis
  PO2( N 50-80 ), PCO2( N 35-45 ), pH( N
  7.25-7.45 )
• PROGNOSTIC INDICES Calculated from ventilator
  parameters and blood gas analysis
• V.I RR MAP PaCO2 lt 1000
  
• MVI RR PIP PaCO2 1000 lt 40 gt 80
• O.I MAP FiO2 PaO2
  lt0.060.175

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TREATMENTAim

• Prevention is better than cure
• Treat the defect
• Reverse the pulmonary hypertension

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Prenatal intervention -open fetal surgery
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Prenatal intervention contdfetoscopic surgery
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Postnatal intervention

• Surfactant
• Nitric oxide
• Sildenafil
• Extracorporeal membrane oxygenation
• Delayed surgery
• Conventional ventilation
• High frequency oscillatory ventilation

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POSTNATAL - SURFACTANT

• Primary surfactant deficiency unlikely
• CDH study group reports an overall potential for
  worse outcome in surfactant treated patients

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POSTNATAL NITRIC OXIDE

• Expected to have a dramatic effect on pulmonary
  hypertension in CDH
• A recent Cochrane review found no clear data to
  support the use of inhaled nitric oxide in
  infants who have CDH

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POSTNATAL - SILDENAFIL

• Decreases pulmonary vascular resistance
• Maybe of some unique benefit but insufficient
  data exists to support its use currently

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POSTNATAL E.C.MO
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POSTNATAL E.C.M.O
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POSTNATAL E.C.M.O

• Rescue therapy after corrective surgery
• Improved survival in CDH patients who had a
  predicted mortality of gt 80
• Now used more for pre-operative stabilisation
• A Cochrane review concluded that ECMO offers
  short term benefits but overall effect of using
  ECMO remains unclear

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POSTNATAL -Delayed Surgery

• Once considered a surgical emergency
• Delay in surgery is not harmful hence there is no
  compelling reason to perform emergent surgery at
  birth
• Now stabilization and delay of surgical repair is
  widely accepted

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POSTNATAL - VENTILATION

• Hyperventilation and induced alkalosis were
  treatment norms in late 80s and 90s
• Gentle ventilation pioneered by Wung and
  colleagues
• Avoid hyperventilation and limit inflation
  pressure to lt 25 cm of water
• Survival rates improved from 40 to 89

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POSTNATAL High frequency oscillatory ventilation

• High survival rates in CDH have been achieved by
  some centers
• Lung protective ventilation must be provided to
  optimize CDH survival

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TREATMENT PROTOCOL Prenatal

• Investigate for associated anomalies
• Ante-natal counseling
• Normal delivery close to term

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TREATMENT PROTOCOL in our hospital

• Naso-gastric tube
• Pre-ductal arterial line
• I/V fluids
• AVOID HYPOTHERMIA, HYPOPERFUSION,HYPOGLYCEMIA AND
  HYPOCALCEMIA
• Endo-tracheal intubation and gentle ventilation
• Sedation

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TREATMENT PROTOCOL in our hospital

• Investigations for anomalies
• Delayed surgery
• Post op ventilation
• Discharge

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SURGICAL ASPECTS
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SURGICAL ASPECTS
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SURGICAL ASPECTS - VATS
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DISCHARGE
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MORGAGNI HERNIA
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Eventration of the diaphragm
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KHALLAS -- SHUKARAN